Linked Life Data

16006796

Source:http://linkedlifedata.com/resource/pubmed/id/16006796

Statements in which the resource exists as a subject.
PredicateObject
rdf:type
lifeskim:mentions
pubmed:issue
8
pubmed:dateCreated
2005-7-11
pubmed:abstractText
More than 85% of Ewing sarcoma/primitive neuroectodermal tumor (ES/PNET), or "Ewing family of tumors" (EFTs), have the translocation, t (11;22) (q24;q12), with others having variant translocations. Identification of these by cytogenetic and/or molecular genetic techniques is specific for EFT and is increasingly recognized as the "gold standard" for diagnosis. However, these techniques are not universally available. We therefore studied a large group of genetically confirmed EFTs to more completely understand the morphologic and immunophenotypic spectrum of this rare sarcoma. Sixty-six cytogenetically, FISH or RT-PCR proven-EFTs were retrieved. In 56 cases, immunohistochemistry (IHC) was performed for pan-cytokeratins (PanCK), high molecular weight cytokeratins (HMWCK), desmin (DES), CD99, CD117, and FLI1 protein using heat-induced epitope retrieval and the Dako Envision system. The cases arose chiefly in children and young adults (median 18 years; range, 3-65 years) of both sexes (male, 32; female, 31; unknown, 3) in a variety of bone (N = 39) and soft tissue (N = 27) sites. Histologically, 46 cases (73%) showed only typical features of ES, 9 cases (16%) showed features of PNET, 3 cases (5%) showed "adamantinoma-like" features, 3 cases (5%) corresponded to "atypical Ewing sarcoma," 3 cases (5%) showed principally intersecting fascicles of spindled cells, and 2 cases had abundant hyalinized matrix. IHC results were as follows: PanCK (18 of 56, 32%), HMWCK (3 of 55, 5%), DES (1 of 56, 2%), CD99 (52 of 52, 100%), CD117 (13 of 54, 24%), and FLI1 (44 of 47, 94%). HMWCK was expressed only in "adamantinoma-like" EFTs, none of which expressed DES. In conclusion, most, but not all, EFTs can be accurately diagnosed using time-honored morphologic criteria and ancillary immunohistochemistry. However, genetic confirmation remains essential for the diagnosis of unusual morphologic variants of EFT, including "adamantinoma-like," spindled, sclerosing, and clear cell/anaplastic variants. Therefore, to exclude or confirm the diagnosis of Ewing's sarcoma in round cell sarcomas having a variety of patterns but not specifically conforming to a tumor of known lineage (eg, rhabdomyosarcoma), cytogenetics, and/or molecular analysis is required.
pubmed:language
eng
pubmed:journal
pubmed:citationSubset
IM
pubmed:chemical
pubmed:status
MEDLINE
pubmed:month
Aug
pubmed:issn
0147-5185
pubmed:author
pubmed:issnType
Print
pubmed:volume
29
pubmed:owner
NLM
pubmed:authorsComplete
Y
pubmed:pagination
1025-33
pubmed:dateRevised
2011-11-17
pubmed:meshHeading
pubmed-meshheading:16006796-Adolescent, pubmed-meshheading:16006796-Adult, pubmed-meshheading:16006796-Aged, pubmed-meshheading:16006796-Antigens, CD, pubmed-meshheading:16006796-Cell Adhesion Molecules, pubmed-meshheading:16006796-Child, pubmed-meshheading:16006796-Child, Preschool, pubmed-meshheading:16006796-Desmin, pubmed-meshheading:16006796-Female, pubmed-meshheading:16006796-Gelsolin, pubmed-meshheading:16006796-Humans, pubmed-meshheading:16006796-Immunophenotyping, pubmed-meshheading:16006796-Keratins, pubmed-meshheading:16006796-Male, pubmed-meshheading:16006796-Microfilament Proteins, pubmed-meshheading:16006796-Middle Aged, pubmed-meshheading:16006796-Proto-Oncogene Proteins c-kit, pubmed-meshheading:16006796-Receptors, Cytoplasmic and Nuclear, pubmed-meshheading:16006796-Sarcoma, pubmed-meshheading:16006796-Sarcoma, Ewing, pubmed-meshheading:16006796-Translocation, Genetic
pubmed:year
2005
pubmed:articleTitle
Morphologic and immunophenotypic diversity in Ewing family tumors: a study of 66 genetically confirmed cases.
pubmed:affiliation
Department of Pathology and Laboratory Medicine, Emory University, Atlanta, GA 30322, USA. afolpe@emory.edu
pubmed:publicationType
Journal Article